Chronic mucocutaneous candidiasis causes frequent or chronic fungal infections of the mouth, scalp, skin, and nails.
To diagnose the disorder, doctors examine a sample from the infected area under a microscope and do blood tests to check for the mutations that cause the immunodeficiency.
Antifungal drugs can usually control the infection, but they must be taken for a long time.
(See also Overview of Immunodeficiency Disorders Overview of Immunodeficiency Disorders Immunodeficiency disorders involve malfunction of the immune system, resulting in infections that develop and recur more frequently, are more severe, and last longer than usual. Immunodeficiency... read more .)
Because T cells T cells One of the body's lines of defense ( immune system) involves white blood cells (leukocytes) that travel through the bloodstream and into tissues, searching for and attacking microorganisms and... read more (a type of lymphocyte) malfunction, the body is less able to fight fungal infections, including infection with Candida (candidiasis Candidiasis Candidiasis is a fungal infection caused by several species of the yeast Candida, especially Candida albicans. The most common type of candidiasis is a superficial infection of... read more ), a yeast. If other parts of the immune system (such as antibodies Antibodies One of the body's lines of defense ( immune system) involves white blood cells (leukocytes) that travel through the bloodstream and into tissues, searching for and attacking microorganisms and... read more ) are functioning, the body may still be able to fight against other infections. However, in some people with this disorder, antibodies also malfunction, making these people susceptible to other infections.
Chronic mucocutaneous candidiasis is due to a mutation in specific genes. Depending on which gene has the mutation, one or two mutations (one from each parent) may be needed to cause the disorder.
Symptoms of Chronic Mucocutaneous Candidiasis
In people with chronic mucocutaneous candidiasis, candidal infections develop and recur or persist, usually beginning during infancy but sometimes during early adulthood.
The fungus may cause mouth infections (thrush Symptoms ) and infections of the scalp, skin, and nails. Membranes lining the mouth, esophagus, digestive tract, eyelids, and vagina (vaginal yeast infection Vaginal Yeast Infection (Candidiasis) A vaginal yeast infection (also called candidiasis) is caused by an infectious organism called Candida, usually Candida albicans. A vaginal yeast infection may cause intense itching... read more ) may also be infected.
In infants, the first symptoms are often thrush that is difficult to treat, diaper rash, or both. Severity varies.
Chronic mucocutaneous candidiasis may cause one or more nails to thicken, crack, and become discolored. A disfiguring rash may cover the face and scalp. The rash is crusted and thick and may ooze. On the scalp, the rash may cause hair to fall out.
Usually, this disorder is chronic, but it does not affect life span.
Many people also have the following:
Endocrine disorders, such as underactive parathyroid glands (hypoparathyroidism Hypoparathyroidism Hypoparathyroidism is a deficiency of parathyroid hormone (PTH) often caused by an autoimmune disorder, treatment-related damage to the parathyroid glands, or removal of the glands during surgery... read more ), diabetes Diabetes Mellitus (DM) Diabetes mellitus is a disorder in which the body does not produce enough or respond normally to insulin, causing blood sugar (glucose) levels to be abnormally high. Urination and thirst are... read more , and underactive adrenal glands (Addison disease Adrenal Insufficiency In adrenal insufficiency, the adrenal glands do not produce enough adrenal hormones. Adrenal insufficiency may be caused by a disorder of the adrenal glands, a disorder of the pituitary gland... read more )
Autoimmune disorders Autoimmune Disorders An autoimmune disorder is a malfunction of the body's immune system that causes the body to attack its own tissues. What triggers an autoimmune disorder is not known. Symptoms vary depending... read more (when the body's immune system malfunctions and attacks the body's own tissues), such as Graves disease Hyperthyroidism Hyperthyroidism is overactivity of the thyroid gland that leads to high levels of thyroid hormones and speeding up of vital body functions. Graves disease is the most common cause of hyperthyroidism... read more
Diagnosis of Chronic Mucocutaneous Candidiasis
Examination of a sample from the infected area under a microscope
Sometimes genetic testing
Doctors suspect Candida infection when people frequently have mouth, scalp, skin, and nail infections with the characteristic skin changes. Examining a sample from the infected area under a microscope and identifying the yeast can confirm that a Candida infection is the cause.
Because people without an immunodeficiency disorder occasionally develop Candida infection, doctors then check for common risk factors for Candida infection, such as diabetes or recent use of antibiotics. If people with frequent Candida infections have no risk factors for Candida infections, the diagnosis is likely to be chronic mucocutaneous candidiasis.
Blood tests to check for a specific genetic mutation can confirm the diagnosis.
Treatment of Chronic Mucocutaneous Candidiasis
Sometimes immune globulin
Usually, the infections of chronic mucocutaneous candidiasis can be controlled with an antifungal drug applied to the skin. If infections persist, they can be effectively treated with fluconazole or another similar antifungal drug taken by mouth. Drugs may have to be taken for a long time.
Immune globulin (antibodies obtained from the blood of people with a normal immune system) is sometimes given. It may be injected into a vein (intravenously) once a month or under the skin (subcutaneously) once a week or once a month.
Endocrine and autoimmune disorders are treated as needed.
Stem cell transplantation Stem Cell Transplantation Stem cell transplantation is the removal of stem cells (undifferentiated cells) from a healthy person and their injection into someone who has a serious blood disorder. (See also Overview of... read more has been used in a few people with specific genetic mutations; however, transplantation is not often used in people with chronic mucocutaneous candidiasis.
The following English-language resource may be useful. Please note that THE MANUAL is not responsible for the content of this resource.
Immune Deficiency Foundation: Other Primary Cellular Immunodeficiencies: General information on chronic mucocutaneous candidiasis and other primary cellular immunodeficiencies, including information on diagnosis and treatment